研究动态
Articles below are published ahead of final publication in an issue. Please cite articles in the following format: authors, (year), title, journal, DOI.

接受肾上腺皮质肿瘤治疗的儿童的青春期、发育和成人身高结果:半个世纪的经验。

Pubertal, Auxological, and Adult Height Outcomes in Children Treated for Adrenocortical Tumors: Half a Century Experience.

发表日期:2024 Aug 22
作者: Fernanda Bora Moletta, Maria Cláudia Schmitt Lobe, Suzana Nesi França, Luiz de Lacerda, Rosana Marques Pereira
来源: Hormone Research in Paediatrics

摘要:

大多数患有肾上腺皮质肿瘤(ACT)的儿童在诊断时表现出生长和骨骼成熟加速,这可能会影响他们的成年身高(AH)。关于 ACT 切除后的生长和青春期模式的知识很少。本研究介绍了接受 ACT 治疗并在巴西单一儿科内分泌服务机构进行随访的患者的青春期和发育发育发育情况。回顾性队列研究包括 1966 年至 2021 年间随访的 63 名患者(47 名女孩)。从 ACT 诊断到发育发育的青春期和发育发育数据分析 AH。 诊断时,患者的骨龄(BA)中位值高于实际年龄(CA),身高标准差评分(H-SDS)大于目标身高(TH-SDS),并且预测成年身高(PAH-SDS) 低于 TH-SDS。 BA和CA之间的差异在随访过程中逐渐减小,并且在肿瘤切除7年后PAH-SDS更加接近TH-SDS。女孩青春期开始的中位 CA 年龄为 9.3 (8.3-11.3) 岁,男孩为 9.9 (9.2-13.6) 岁。 9 名患者(6 名女孩)出现中枢性性早熟 (CPP),这是受到 CA 的影响
Most children with adrenocortical tumors (ACTs) present with accelerated growth and skeletal maturation at diagnosis, which potentially compromises their adult heights (AHs). Knowledge about growth and pubertal patterns after ACT resection is scarce. This study presents the pubertal and auxological development of patients treated for ACT and followed up at a single pediatric endocrinology service in Brazil.Retrospective cohort study including 63 patients (47 girls) followed up between 1966 and 2021. Pubertal and auxological data from ACT diagnosis to AH were analyzed.At diagnosis, the patients had median values of bone age (BA) more advanced than chronological age (CA), height standard deviation score (H-SDS) greater than target height (TH-SDS), and predicted adult height (PAH-SDS) lower than TH-SDS. The difference between BA and CA decreased gradually during follow-up and the PAH-SDS moved closer to the TH-SDS 7 years after tumor resection. Puberty started at a median CA of 9.3 (8.3-11.3) years in girls and 9.9 (9.2-13.6) years in boys. Nine patients (6 girls) developed central precocious puberty (CPP), which was influenced by a CA > 4 years at diagnosis. The difference between AH-SDS and TH-SDS was not significant (p=0.3). The factors independently associated with AH below TH were CA > 4 years at diagnosis, time between clinical manifestation and diagnosis > 1 year, and development of CPP.Most patients treated for ACT during childhood attained AH within the TH despite presenting with advanced skeletal maturation at diagnosis. Development of CPP was not infrequent.S. Karger AG, Basel.